Screening for associated conditions and comorbidities in children with sickle cell disease

Condition/comorbidity	Screening test(s) used	Age at which screening is started	Action(s) to take if screening is abnormal
General pediatric issues that may require special attention in patients with SCD (assess at all routine visits)
Asthma	Symptom screening by ROS History of recurrent wheezing and/or recurrent ACS	Infancy	Referral to an asthma specialist for spirometry and to optimize asthma management
Depression and other mental health issues	Screening questions or questionnaires	Once old enough to answer screening questions, typically by school age	Referral to mental health provider
Growth failure and delayed puberty	Measurement of height, weight, and assessment of pubertal stage	Infancy	Referral to endocrinologist
Hypertension	Manual or automated blood pressure measurement	Infancy	Referral to nephrologist
OSA	Screening questions regarding frequency of OSA symptoms	Age 2 years	Refer to pulmonary/sleep medicine specialist for PSG If PSG confirms OSA, refer to otolaryngologist for possible adenotonsillectomy
SCD-specific issues (assess at least annually)
Nephropathy	Measurement of urinary protein (eg, urinalysis or urine protein-to-creatinine ratio) Serum creatinine	By age 10 years*	Referral to nephrology
Pulmonary hypertension	Echocardiogram	Once between age 8 to 18 years^¶	Referral to cardiology
Retinopathy	Dilated eye examination	10 years	Referral to ophthalmology
Stroke	TCD (patients with Hb SS or Hb S-beta⁰ thalassemia only) Assess for neurocognitive or other neurologic deficits (eg, developmental delay, marked decline in school performance, frequent headaches)	2 years^Δ	Obtain MRI/MRA if TCD is abnormal or if there are clinical concerns Referral to neurology for patients with confirmed stroke Follow-up abnormal TCD results with repeat testing^Δ For persistently abnormal flow velocity on TCD, primary prophylaxis with chronic transfusion is generally indicated^Δ

SCD: sickle cell disease; ROS: review of symptoms; ACS: acute chest syndrome; OSA: obstructive sleep apnea; PSG: polysomnography; TCD: transcranial Doppler; Hb: hemoglobin; MRI: magnetic resonance imaging, MRA: magnetic resonance angiography.
* The optimal screening test and intervals for testing for sickle cell nephropathy are uncertain. We typically begin annual screening at age 10 years with a spot urine protein-to-creatinine ratio and serum creatinine level. Other experts start screening as early as age 3 years.
¶ There is disagreement among experts, including UpToDate authors, about the benefit and timing of the screening echocardiogram in asymptomatic adolescents with SCD. An echocardiogram should be performed in patients who develop symptoms suggestive of pulmonary hypertension (eg, exercise intolerance, chest pain, or otherwise unexplained respiratory symptoms). In the absence of concerning symptoms, it is reasonable to obtain a screening echocardiogram once during adolescence. Refer to UpToDate topic on pulmonary hypertension in SCD for additional details.
Δ For children with Hb SS or Hb S-beta⁰ thalassemia, TCD screening begins at age 2 years and continues through age 16 years (if feasible). Screening is not required for individuals with other forms of SCD (eg, Hb SC, Hb S-beta⁺ thalassemia), although it may be appropriate in those with evidence of severe disease such as frequent episodes of acute chest syndrome or vaso-occlusive pain episodes. If performed, it should be noted that the risk stratification parameters established for patients with Hb SS may not apply to those with other genotypes. Refer to UpToDate topic on prevention of stroke in SCD for additional details of the interval of follow-up for abnormal TCD results and indications for chronic transfusion therapy.

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Screening for associated conditions and comorbidities in children with sickle cell disease